Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood

Abstract

Chronic bullous disease of childhood (CBDC) is the most common acquired autoimmune blistering disorder of childhood and is characterized by linear IgA staining of the basement membrane zone on direct immunofluorescence. This autoimmune attack on structural proteins, usually proteolytic fragments of collagen XVII, renders the dermal-epidermal junction prone to blistering. Diagnosis is confirmed by characteristic histology and direct immunofluorescence. Prognosis is generally favorable, with spontaneous remission usually occurring by puberty; however, cases with severe morbidity and cases persisting into adulthood have been reported. This article discusses the clinical features, diagnosis, and pathogenesis of CBDC in more detail.